Guillain Barré Syndrome

GBS/CIDP described

GBS is a syndrome; i.e. it is a collection of symptoms and signs. It is not a single disease, but rather consists of serveral related diseases. It is a rare disorder affecting 1–2 people per 100,000 fairly consistently around the world. This implies that about 40 to 80 New Zealanders are affected each year although recent research has suggested this may be as high as 100 annually. All of these diseases have in common an immune attack on the components of the peripheral motor sensory nervous system resulting in rapidly progressive weakness, sometimes progressing to severe or even complete paralysis. Progression of the disease always stops within 4 weeks and there is a prolonged recovery phase.

The most common form of GBS is called acute (short-lasting) inflammatory demyelinating polyneuropathy (AIDP) because the immune attack is directed at the myelin sheath that surrounds and insulates the individual nerve fibres that are called axons.

A rare form of GBS (accounting for about 5% of all attacks) is called Miller Fisher syndrome (MFS). In this form of GBS the immune attack is directed against the nerves that control eye movement and coordination, leading to double vision (diplopia), droopy eyelids (ptosis) and incoordination and imbalance (ataxia).

The immune attack is occasionally directed against the axon itself. If only motor axons are affected the syndrome is called acute motor axonal neuropathy (AMAN) and if both motor and sensory axons are affected it is called acute motor and sensory axonal neuropathy (AMSAN) – this is probably just a more severe form of AMAN.

CIDP is a chronic or ongoing neuropathy that closely resembles GBS; the main difference is the pace of progression. Instead of evolving over 4 weeks it evolves much more slowly, typically over a period of months but occasionally over many years. It is also a disease in which there is an immune attack directed against the myelin sheath. Whether there is a chronic disease corresponding to AMAM and AMSAN is controversial although it is recognised that there is a chronic disease akin to MFS called CANOMAD.

Other, very rare, chronic demyelinating neuropatghies that have some resemblance to CIDP include multifocal motor neuropathy (MMN, sometimes known as Parry’s syndrome), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM, also known as Lewis–Sumner syndrome) and paraproteinemic neuropathy.

Whilst further attacks of GBS are very few and far between, CIDP relapses are more common. The term autoimmune disease is also applied to GBS/CIDP, which means that the body’s own defence mechanism turns on itself. The precise reasons why this happens are still the subject of research but it appears that the lymphocytes (the cells that defend the body), possibly already called into action by a previous infection, attack the myelin sheath insulator of the nerves or more rarely the nerves (axons) themselves by mistake.

Fatalities arising from GBS attacks are rare. Those that do occur are usually caused from complications arising from respiratory failure and infections rather than from GBS itself.

Onset of GBS

Onset of GBS symptoms frequently follows some sort of health problem. The most common examples are food poisoning (campylobacter), flu, viral infections, childbirth, surgery or vaccinations.

The illness can occur at any age, although it is slightly more common among the elderly and also more common among men than women. It is not hereditary and is not infectious or contagious.

The interval from onset to peak disability can vary from hours – more usual with GBS – to weeks – often the case with CIDP. About 60% of cases will be unable to walk at the height of the illness. Respiratory function will be impaired in some 50% of cases, with 20% to 30% requiring assistance with ventilation or other intensive care, although this is less common with the CIDP variant.

Symptoms

The first symptoms are usually tingling (pins and needles) or numbness starting first in the feet and rising to the hands and sometimes the face. Unlike a stroke, which typically affects only one side of the body, GBS usually causes altered sensations and weaknesses on both sides. The legs may feel heavy, wooden and weak and the arms may feel limp. Gripping, turning or holding things may be difficult. Rising from a chair may become a problem. In the early stages the symptoms can be vague and puzzling and it may be hard to convince others that there really is something wrong.

These symptoms may remain mild and clear up rapidly but a more common result, at least in the case of a GBS attack, is that they deteriorate and hospital admission becomes necessary. Patients may become progressively weaker until they are unable to stand and their tendon reflexes disappear. The condition usually peaks within two to three weeks. After that there is a “plateau phase” that lasts anything from a few days to several weeks, where the condition gets neither better nor worse. Most patients are so weak at this time that they are usually confined to bed and need to rest. Some patients may suffer a lot of pain arising from the inflammation of the nerves, from muscles that have temporarily lost their nerve supply, from stiff joints or because of long periods of having to lie or sit down.

Diagnosis

Diagnosis is usually on clinical grounds – the doctor recognises the condition by the symptoms and their effects on the body. Blood tests, spinal fluid tests and an electromyogram that measures electrical activity within the muscles can help confirm the clinical diagnosis.

Treatment

The is no prevention or cure as such for GBS and it usually gets better on its own over time with the demyelinated nerves repairing themselves. No drugs have been proven to make any difference to the speed of recovery, but good nursing and, for the more severe cases, early treatment using one of the two available blood related treatments, may shorten the duration of the illness and improve the ultimate outcome for the patient.

The two treatments are plasma exchange (plasmaphereses) where over a period of days the patient's blood is passed through a machine where the antibodies in the plasma that are attacking the nerves are removed. The alternative is gamma globulin (also called immunoglobulin – IvIg), which is a human blood product and is given intravenously over a period of days. This treatment is expensive but more simple than plasma exchange.

CIDP patients can also benefit from the use of some drugs such as steroids and azathioprine, which aim to suppress the damaging autoimmune reaction, though this has to be balanced against the risk of suppressing the normal immune response to infections.

Physiotherapy is vital to minimise muscle wasting and to regain muscle strength and activity, but exercise must be balanced with the need for rest.

Recovery can take anything from a few weeks to several months – the average is 3 to 6 months. Two thirds of those who suffer from GBS recover completely. The remainder can be left with varying degrees of weakness and discomfort. In most cases this does not affect people's lives seriously. A few however are unable to go back to their former occupation because of residual disabilities, for example, hand weakness, foot drop or the need for walking aids. In these cases long term rehabilitation may be required.

Further Information

The group has available a number of booklets, detailed below, which are available free of charge to interested parties. Please contact our National Co-ordinator, Tony Pearson for copies of these publications: (03) 540 3217

What is GBS?

CIDP

Recovery Advice

A Guide for Carers

Physiotherapy

IVIG in the Treatment of Acute and Chronic Neuropathies

Guillain Barre Syndrome and Pregnancy

Childhood Guillain Barre Syndrome – A guide for parents and carers.

Caring for a child with GBS – A personal account from a mother caring for a child with CIDP.

Teen and Young Adult Handbook – A personal account from a teen with GBS.

The Group also has available CD guides, again free of charge, explaining GBS and CIDP.

We are grateful for the assistance given by the UK GBS Support Group and the GBS/CIDP Foundation International for the use of their material and permission to distribute the literature in New Zealand.

A number of books have been written by former GBS sufferers including

No Laughing Matter by Joseph Heller and Speed VogelBed No. 10 by Sue Baier and Mary Zimmeth SchamakerA First Step by Brian S LangtonLearning to Walk Again by Anne K BrandtNumb Toes and Aching Soles: Coping with Peripheral Neuropathy by John A SenneffThe Darkness is not Dark by Regina R RothUp from the Abyss by Italo GiovanniThe “Official” Patients Sourcebook of Guillain Barré Syndrome by Ikon Health Publications

Most are of these are available from Amazon.com.

GBS – An Overview for the Lay Person by J.S. SteinbergCaring for a Child with GBS by P. Schard

Both available from GBS/CIDP Foundation International.

Perhaps the most useful book, and certainly the most recent, detailing the issues associated with GBS and CIDP is

Guillain–Barré Syndrome: From Diagnosis to Recovery

Written by our own medical advisor Dr Gareth Parry and his colleague Joel Steinberg. Published by the American Academy of Neurology it is available from the Group for NZ$30 – contact Tony if you would like to buy a copy.

A review of the book appears in the September 2007 Newsletter.

History of Guillain Barré syndrome

Georges Guillain

Jean-Alexandre Barré

Guillain Barré syndrome was discovered by the French physicians Jean-Alexandre Barré, Georges Guillain and Andre Strohl in the early 1900s following tests on soldiers returning from World War 1. Many cases of an identical condition had been described over the preceding 80 years but these neurologists identified one of the characterising features of the disease – the increased concentration of protein in the spinal fluid without evidence of inflammation.